This neoplasm is not reportable.
Name
Disseminated juvenile xanthogranuloma
Reportability
This neoplasm is not reportable
Abstractor Notes
The skin and soft tissues are affected most commonly. Disseminated forms commonly affect the mucosal surfaces, in particular with the upper aerodigestive tract.
There is known associations with Neurofibromatosis type 1 (NF1). If patient has both JXG and NFI, they are at a higher risk of juvenile myelomonocytic leukemia (JMML).
Treatments used for LCH are usually used.
For more information, see the Histiocytosis Association website (http://www.histio.org/page.aspx?pid=391)
There is known associations with Neurofibromatosis type 1 (NF1). If patient has both JXG and NFI, they are at a higher risk of juvenile myelomonocytic leukemia (JMML).
Treatments used for LCH are usually used.
For more information, see the Histiocytosis Association website (http://www.histio.org/page.aspx?pid=391)
Alternate Names
Benign cephalic histiocytosis
Deep JXG (if soft tissue involvement)
Generalized (non-lipidemic) eruptive histiocytosis
Progressive nodular histiocytosis
Definition
Disseminated juvenile xanthogranuloma (JXG) is characterized by a proliferation of histiocytes similar to those of the dermal JXG, commonly having a foamy (xanthomatous) component with Touton-type giant cells. There is evidence for clonality in some instances.
Solitary dermal (skin) JXG is more common then other forms and normally does not progress to disseminated forms. The majority of the disseminated forms occur by the age of 10 years, over half within the first 6 months of life.
For the adult form of this disease, see Erdheim-Chester disease.
Solitary dermal (skin) JXG is more common then other forms and normally does not progress to disseminated forms. The majority of the disseminated forms occur by the age of 10 years, over half within the first 6 months of life.
For the adult form of this disease, see Erdheim-Chester disease.
Immunophenotyping
This data item does not apply
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 480
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 480